INTRODUCTION:Acute infectious purpura fulminans, one of three variants of purpura fulminans (PF), is seen most commonly in children and is associated with streptococcal, pneumonococcal, meningococcal and viral infections. Herein a case is reported in an adult with streptococcal infection 30 years post splenectomy for lymphoma. This case highlights the most common cause of acute infectious purpura fulminans seen in the adult population and in patients with hyposplenia and aspelnia, and correlates the clinical presentation with pathologic and radiologic images.
CASE PRESENTATION:This is a 61 year-old man with remote history of lymphoma 30 years ago that was staged with splenectomy. The patient received pneumococcal vaccination with in the last five years, but he did not receive vaccination prior to his splenectomy. He presented to the Emergency Department complaining of malaise with skin rash on his face and legs of a few hours duration. Then the patient developed progressive shortness of breath and was severely hypoxemic (PaO2 of 45 mmHg on room air) upon presentation to the hospital. Empirical broad spectrum antibiotics were started after obtaining blood cultures.His condition deteriorated very rapidly with progression to cardiac arrest within minutes of arrival. He was successfully resuscitated, mechanically ventilated, and placed on vasopressors for circulatory collapse. His chest x-ray and chest computed tomography showed bilateral air space disease. Blood cultures grew Streptococcus Pneumoniae. Laboratory studies reveald disseminated intravascular coagulation (DIC). His condition declined rapidly over 24 hours to septic shock with multi-organ failure. Over the next 48 hours, the rash on his face and legs evolved from purpuric macules to skin necrosis and dry gangrene for his toes. A biopsy of a lesion on the wrist was performed, which showed multiple non-inflammatory thrombi in many capillaries and venules with ischemic loss of epidermis above the basilar layer. These skin finding were consistent with a clinical diagnosis of Acute Infectious Purpura. His condition stabilized in the intesive care unit (ICU), but he required continued mechanical ventilation and hemodialysis and had evidence of multi-infarct brain injury from DIC. The patient never regained a meaningful neurologic function and was discharged to a long term care facility.
DISCUSSIONS:Neisseria meningitidis infection is the leading cause for acute infectious PF, but S pneumoniae is not an uncommon etiologic agent, especially in the adult population and in asplenic individuals. Asplenic or functionally hyposplenic patients are known to be at high risk for infections with encapsulated bacteria such as S pneumoniae, group A streptococcus, Haemophilus influenzae type b, and Neisseria meningitidis. One of the characteristic cutaneous findings of early PF is the presence of erythematous or purpuric macules in the acral sites, such as the nose, ears, cheeks, fingers, and toes. Within 48 hours these purpuric macules evolve into symmetric peripheral gangrene.These data suggest that in PF the presence of asplenism should alert the clinician for an infectious etiology, especially with encapsulated bacteria. Because Purpura Fulminans is not a specific disease, the management must be toward treating the underlying infection and the supportive care. However, The initiation of appropriate treatment early in the course of the disease may be lifesaving in this usually fatal condition. the mortality rate for this disease remains high at 40% (range, 20–70%).
CONCLUSION:Acute infectious purpura fulminans is a rapidly progressive syndrome of hemorrhagic skin necrosis associated with acute infection, disseminated intravascular coagulation, and multi-organ failure. S pneumoniae is not an uncommon etiologic agent, especially in the adult population and in asplenic individuals. The physician should be aware of the early cutaneous findings of purpura fulminans (PF) to establish an accurate diagnosis of the underlying condition and initiate appropriate treatment early in the course of the disease.
DISCLOSURE:Mohammad Al-Hamed, No Financial Disclosure Information; No Product/Research Disclosure Information